Sumantra Sarkar

Growth of children with juvenile idiopathic arthritis

ObjectiveTo evaluate the growth pattern in children with juvenile idiopathic arthritis and its subtypes in comparison with age, sex and temporally matched controls.Study designProspective study.SettingPediatric rheumatology clinic of a tertiary care hospital in Eastern part of India.ParticipantsSeventy-five children (2–12 years) diagnosed as juvenile idiopathic erthritis by International League of Associations for Rheumatology criteria and 75 age- and sexmatched controls.InterventionWeight, height and body mass index were recorded at six monthly interval in both groups over a period of 3 years.Main outcome measuresweight, height and body mass index.ResultsSubtype distribution of juvenile idiopathic arthritis was: oligoarthritis (49%, n=37), rheumatoid factor negative polyarthritis (27%, n=20), rheumatoid factor positive polyarthritis (8%, n=6), systemic onset (15%, n=11) and enthesitis related arthritis (1.3%, n=1). Anthropometric parameters in children with juvenile idiopathic arthritis were not significant different from controls. Comparison between the subtypes showed significant differences in height (P=0.011), weight (P=0.005), and growth velocity (P=0.005), but not in body mass index. Systemic onset disease led to significant restriction in height (P=0.018; 95% CI 2.13–33.77) and weight (P=0.008; 95% CI 1.47–14.43) compared to controls. Growth velocity was significantly affected in rheumatoid factor positive polyarthritis (P=0.003; 95% CIO. 46–3.14).ConclusionsChildren with juvenile idiopathic arthritis do not have significantly lower values of anthropometric parameters compared to controls. Significant restriction in height and weight is seen in systemic onset disease, and growth velocity is significantly reduced in rheumatoid factor positive subjects.

Acute stress-related psychological impact in children following devastating natural disaster, the Sikkim earthquake (2011), India

Background: Psychological stress following natural disaster is common. Despite several earthquakes in India, data on evaluation of acute stress among the child victims in the early postdisaster period is scarce. Immediately following a devastating earthquake (6.9 Richter) at Sikkim on September, 18 2011, many children attended North Bengal Medical College, the nearest government tertiary care institution, with unusual stress symptoms. Objective: Evaluation of acute stress symptoms in children in the immediate postearthquake period. Materials and Methods: This was a cross-sectional study done over 4 weeks and includes all the children from 1 to 12 years presenting with unusual physical or behavioral symptoms. Those with major injuries requiring admission were excluded. They were divided into two age groups. For older children (8-12 years) the 8-item Children Impact of Event Scale (CIES) was used for screening of stress. Unusual symptoms were recorded in younger children (1-8 years) as CIES is not validated < 8 years. Result: A total of 84 children (2.66%) out of 3154 had stress symptoms. Maximum attendance was noted in first 3 days (65.47%) and declined gradually. In children ≥ 8 years, 48.78% had psychological stress, which was statistically significant on CIES scores without any gender predilection. Static posturing (41.86%), sleeplessness (32.55%), anorexia (9.30%), recurrent vomiting (13.95%), excessive crying (13.95%), or night-awakenings (4.65%) were found in younger children (n = 43) and three required admission. Conclusion: This study represent the first Indian data showing statistically significant psychological impact in older children (8-12 years) and various forms of physical stress symptoms in young children (1-8 years) following earthquake.

Tacrolimus Induced Diabetic Ketoacidosis in Nephrotic Syndrome

Tacrolimus, a reversible calcineurin inhibitor, is known for its diabetogenic potential. The incidence of diabetes is less frequent among the patients of nephrotic syndrome in comparison to organ transplant recipients. Diabetic ketoacidosis (DKA) is even rarer. DKA as the first presentation of new onset tacrolimus induced transient type 1 diabetes despite a lower dose range and low trough level of the drug is being reported in a 12-y-old girl with steroid resistant nephrotic syndrome.

A pediatric case series of abdominal epilepsy

BackgroundAbdominal epilepsy (AE) is an infrequent cause of recurrent abdominal pain in children. It is characterized by paroxysmal episodes of abdominal pain, a variety of other abdominal complaints, electroencephalogram abnormalities, and response to anti-epileptic agents. We described the clinical profile of six patients with AE.MethodsWe conducted a retrospective survey of AE in children from the records of the hospital. The diagnosis of AE was dependent on recurrent abdominal symptoms, subtle central nervous system abnormalities, electroencephalogram abnormalities and response to anticonvulsant agents.ResultsThe six patients were diagnosed with AE. The incidence of the disease was 0.07% in all admissions to the pediatric ward. Recurrent pain was common in all patients except two who had additional recurrent vomiting. In this series, the boy to girl ratio (1:2) was unequal.ConclusionHigh suspicion is required for the diagnosis of AE after exclusion of other possible causes.

Generalized hyperpigmentation in Wilson's disease: An unusual association.

Wilsons disease, an autosomal recessive disorder of copper metabolism, most commonly presents either with hepatic or neurological features. But, it may sometimes have certain atypical presentations posing diagnostic difficulties. We report here a case of Wilsons disease presenting with generalized hyperpigmentation of skin who also developed neurological manifestations subsequently. We aim to highlight the importance of keeping Wilsons disease as one of the differentials in patients who present with hyperpigmentation and neurological symptoms compatible with copper deposits in the central nervous system and proceed for investigations accordingly.

Phenytoin induced life threatening macroglossia in a child

Isolated acquired macroglossia of tongue rarely reported. It occurs due to causes like hereditary angioedema, localized angioedema, etc., Here we describe an 8-year-old boy developing life threatening localized angioedema of tongue due to phenytoin without any association with drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome or pseudolymphoma encountered in rural medical college. Anticonvulsants, that is, phenytoin induced this isolated peculiar complication, which was not described before.

Organophosphorus-induced extrapyramidal intermediate syndrome in an adolescent suicide attempt survivor

The victims of organophosphorus (OP) pesticide poisoning usually present with acute cholinergic crisis, due to the inhibition of the enzyme acetylcholinesterase. Any neurological complication in the form of intermediate syndrome is rare and its presentation with extrapyramidal symptoms is even rarer. The authors report such a case in a 12-year-old adolescent girl, who survived a near lethal suicidal attempt.

Karate Related Injury- Are We Prepared?

To the Editor: Growing interest in martial arts training is a welcome trend because of its’ multifaceted advantages. But, while encouraging our children to undergo this training we should be watchful of the possible life threatening events. A 10-y-old girl was admitted with acute onset severe pain and restriction of movement of neck following a punching injury during a karate practice session. There was no neurodeficit. She did not have any predisposing condition for atlanto-axial instability (AAI). X rays and MRI scan demonstrated atlanto-axial subluxation with backward bending of odontoid process and kinking of medulla oblongata. The atlanlanto-axial distance in the anteroposterior plane was 6 mm (Fig. 1). As martial arts coaching centers are coming up, we need to introspect the preparedness to deal with such accidental injuries. Karate not only strengthens physical and psychological fitness, but also has a role in self-defense. Efforts are on by the authorities to train girls in martial arts in the light of recent spurt in crime against women [1]. Although we could find a few studies from other countries on incidence of martial art related injuries [2, 3], there is hardly any from India. In a study on injury incidence in 3 types of martial artskarate, taekwondo, and judo, most injuries were attributed to karate (79.5 %) [4]. However, we could not find any specific information on the incidence of atlanto-axial subluxation nor could we find any guidelines regarding screening for atlanto-axial instability before imparting martial arts training. There is an urgent need to frame regulatory guidelines, regarding the need of expertise to anticipate and manage accidents. Pre-training screening guidelines for eligibility to undergo the physical demands of martial arts training and an injury surveillance system should be in place. The American Academy of Pediatrics (AAP) guidelines for eligibility for sports participation in certain medical conditions is a welcome step in this direction [5]. It is high time the Indian Academy of Pediatrics also formulates recommendations for our population. We aim to stimulate thoughts of fellow clinicians towards formulation of such guidelines so that Fig. 1 Sagittal section of T1 weighted image (T1WI) showing widening of atalanto axial distance more than 6 mm with Dens compressing the cortico-medullary junction resulting in kinking of spinal cord

Necrotic Tongue Ulcer: An Unusual Presentation of Childhood Polyarteritis Nodosa

To the Editor: Polyarteritis nodosa (PAN), an uncommon form of childhood vasculitis, may be associated with various atypical presentations [1, 2]. Necrotic tongue ulcer as a presenting manifestation is rare and reported here for the first time from India. An 8-y-old girl, apparently well before, presented with necrotic ulcerative lesion over the tip and dorsum of the tongue (Fig. 1) associated with fever and severe myalgia. The lesion progressed in next 2 d. An erythematous maculopapular rash appeared over the anterior aspect of legs on day three. This was followed by pain and swelling in her left wrist and ankle joints suggestive of arthritis. Her blood pressure (BP) was recorded as 130/90 mmHg. Peripheral pulses were equally palpable. Investigations revealed hemoglobin 10.80 g/dL, total leucocyte count 22,370/cmm with polymorphs 65 %, lymphocytes 30 %, monocytes 3 %, eosinophils 2 % and Platelets 4.6 lacs/cmm. ESR was 68 mm 1st h and C-reactive protein was 26 mg/dL (normal <6 mg/dL). Urine examination showed albumin-trace, plenty of red blood cells and hyaline cast with 24 hprotein excretion 86 mg. Urea, creatinine and electrolytes were normal. Liver enzymes were mildly elevated. HBsAg was negative. Creatinine phosphokinase (CPK) was 220 U/L (normal <150 U/L). Rheumatoid factor (RF), anti-nuclear antibodies (ANA) were negative and C3 was normal. Indirect immunofluorescence microscopy showed a peri-nuclear staining pattern by antibodies directed against neutrophil cytoplasmic antigen. Anti-neutrophil cytoplasmic antibody (ANCA) against the myeloperoxidase (MPO) of the neutrophils (ELISA) was negative. Chest X-ray, ECG, echocardiography and sonography of abdomen were normal. Sonography of ankles revealed fluid accumulation. Skin biopsy showed infiltration of the medium sized vessels with polymorphs interposed with fibrinoid necrosis suggestive of necrotising vasculitis. CT angiogram of abdominal vasculature including renal vessels was inconclusive. She was put on amlodipine (0.1 mg/kg), topical anaesthetics gel and antibiotics. Dramatic healing of tongue ulcer was noted following prednisolone (2 mg/kg/d) (Fig. 2). She did well on follow up and prednisolone was tapered gradually. R. Mondal :K. A. Khan :A. Roy Pediatric Rheumatology Clinic, Department of Pediatric Medicine, North Bengal Medical College and Hospital, Darjeeling, India

Congestive Heart Failure: An Uncommon Presentation of Systemic Onset Juvenile Idiopathic Arthritis (SOJIA)

Children with systemic onset Juvenile idiopathic arthritis (SOJIA) are known to develop myocarditis and congestive heart failure as a complication of disease process infrequently. Cardiac involvement causing congestive heart failure as a presenting manifestation in SOJIA is rarely described in the literature. The authors describe a case of an 11- yr- old boy with SOJIA who presented with congestive heart failure following active myocarditis, with the flare of the disease process. The cardiac manifestations, along with the disease activity were controlled with intensive immunosuppressive therapy.